Anti-ATXN3L Rabbit Polyclonal Antibody (Alexa Fluor® 647)

Supplier: Bioss

BS-4807R-A647
10404-622EA 811.1 USD
10404-622
Anti-ATXN3L Rabbit Polyclonal Antibody (Alexa Fluor® 647)
Antibodies
Defects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3) ; also known as Machado-Joseph disease (MJD). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.

Recommended Dilutions: IF(IHC-P): 1:50-200

Type: Primary
Antigen: ATXN3L
Clonality: Polyclonal
Clone:
Conjugation: Alexa Fluor® 647
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human
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